Myelodysplastic syndrome with increased marrow fibrosis: a distinct clinico‐pathological entity

Abstract

Seventeen cases of myelodysplastic syndrome (10 primary and seven secondary to previous radio-chemotherapy), characterized by trilineage dysplasia, severe bone marrow fibrosis and a high number of megakaryocytes, are described. All of these patients had similar clinical and prognostic features consisting of pancytopenia, modest or absent visceral enlargement and poor survival. The use of CD61 antibodies, which recognize megakaryocytic cells at all stages of maturation, confirmed that these patients had a higher number of these cells than either normal subjects or patients affected by myelodysplastic syndrome (MDS) without fibrosis. Furthermore, primary and secondary MDS with fibrosis, although clinically and histopathologically similar, differed in terms of the number of megakaryoblasts which were significantly higher in primary forms (P less than 0.02). We conclude that MDS with fibrosis may represent a clinicopathological entity which needs to be distinguished from other MDS subtypes as well as from idiopathic myelofibrosis or malignant myelosclerosis.

Acute panmyelosis with myelofibrosis: an entity distinct from acute megakaryoblastic leukemia

“…The distinction between APMF and aggressive subtypes of MDS with fibrosis (MDS-F) 25,33 such as refractory anemia with excess of blasts type 2À with fibrosis, in particular, may be difficult, as also illustrated by our case 3. These difficulties have been outlined in published reports, 22,47,48 One of the reason concerns the degree of myelofibrosis which, in both conditions, shows significant overlap.…”

“…8,[18][19][20][21] Yet others believe that it is an acute variant of MDS, 22-24 a group of disorders in which myelofibrosis can also be observed. 25,26 The primary differential diagnosis of APMF is, however, with AMKL.…”

“…These difficulties have been outlined in published reports, 22,47,48 One of the reason concerns the degree of myelofibrosis which, in both conditions, shows significant overlap. However, important clinical differences exist between the two conditions, particularly the abrupt onset for APMF and its shorter survival rate in comparison with MDS-F. 22,25 The latter condition appears clinically heterogeneous and at least in one study has been associated with comparatively long survivals. 49 It seems therefore prudent to retain the diagnostic terms separately, and to consider APMF a variant of AML as defined by the WHO system.…”

Bone marrow fibrosis in patients with primary myelodysplastic syndromes has prognostic value using current therapies and new risk stratification systems

“…1,2 The clinical importance of bone marrow fibrosis in patients with myelodysplastic syndromes, however, has not been well recognized in the past and this histological feature was not incorporated into the 2008 World Health Organization classification system. 3 In recent years, bone marrow fibrosis has been increasingly recognized as an adverse feature in patients with myelodysplastic syndromes, which correlates with increased risk of early bone marrow failure, transformation to acute myeloid leukemia, and an inferior patient outcome.…”

Emergence of JAK2-mutant primary myelofibrosis in myelodysplastic syndrome: rare case report, literature review, and implications for clonal progression

“…Although BM fibrosis can be seen in about 10-15% MDS patients and is a poor prognostic indicator, it is found more commonly in higher-risk cases and therefore only occasionally in RA [2][3][4][5][6][7]. Accordingly, recognizing that most cases of BM fibrosis occur in refractory anemia with excess (5-19%) blasts (RAEB) MDS, the current World Health Organization (WHO) Classification includes the provisional entry, RAEB with fibrosis (RAEB-F).…”